[PAGID] patient who lacks long-term memory antibody responses

[Soheil Chegini]

Jason,
 
In some patients after repair of congenital cardiac anomalies elevated
right atrial pressure and secondary intestinal lymphangiectasia
develops.  They typically have protein losing enteropathy and suppressed
immunoglobuli levels and T-cells, primarily CD4.  The giveaways are
often diarrhea and global hypoproteinemia, but some patients can
compensate fairly well and have normal albumin and no overt GI symptoms.
 Elevated fecal alpha-1 antitrypsin will establish the diagnosis.
 
Best,
Soheil
 
Soheil Chegini, MD
Assistant Professor of Medicine & Pediatrics
Penn State Hershey Medical Center
500 University Dr. H041
Hershey, PA 17033
Tel. 717-531-6525
Fax 717-531-5785



>>> <raas0027 at umn.edu> 7/17/2009 10:49 AM >>>

I have a 4 year old patient with similar prophile. Hx tetrology of
fallot 
at birth with several corrective surgeries over the first 2 years. A
thymus 
was present and removed with the first thoracotomy. Chromosomal studies
and 
clinical picture make DiGeorge syndrome unlikely.

She has had chronic purulent bronchitis. Despite inhaled
corticosteroids, 
mucolytics, vest therapy and rotational antibiotics, frequent
therapeutic 
bronchoscopies were the only thing providing improvement. Bronchoscopy
has 
revealed mostly encapsulated organisms.

Immunologic evaluation unremarkable except for mild T-cell lymphopenia

(often seen in thymectomized infants?).

Excellent initial response to Pneumovax, however titers dropped to <1.0

within 9 months.

She was started on Vivaglobin and in two months has had dramatic 
improvement. Off vest, mucolytics, inhaled steroids and antibiotics. 
Parents and pulmonologists are thrilled. We'll see how she does this 
winter.


Good discussion topic.

-Jason

On Jul 17 2009, Howard M Lederman wrote:


> I have a fascinating 12 yr old male patient with a history of

recurrent 

> infections (mostly upper and lower respiratory tract). No individual



> infection has been overwhelmingly severe, but the sum of all

infections 

> leads to a significant number of school absences.He has low IgG and

IgM 

> levels with normal IgA levels. He makes normal IgG antibody responses

to 

> a wide variety of vaccines, but does not seem to be able to maintain



> long-term immunity. (See attached spreadsheet. Sheet 1 tabulates the



> antibody and immunglobulin levels; sheet 2 lists the

immunizations.)Is 

> this a pattern that others have seen? I am open to any ideas for

further 

> evaluation. Would anyone offer this child gamma globulin? Howard

Howard 

> M. Lederman, M.D., Ph.D. Professor of Pediatrics, Medicine and

Pathology 

> Division of Pediatric Allergy and Immunology Johns Hopkins Hospital -



> CMSC 1102

>600 N. Wolfe Street 

>Baltimore, MD 21287-3923 

>Phone: 410-955-5883 

>Fax: 410-955-0229 

>Email: Hlederm1 at jhem.jhmi.edu 

>

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-- 


Jason P. Raasch, MD

Midwest Immunology Clinic
City Center Professional Building
15700 37th St NE
Suite 110
Plymouth, MN 55446

Office: 763.577.0008
FAX: 763.577.0192
raas0027 at umn.edu 

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